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paraproteinemias/epileptischer anfall
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Monoclonal Gammopathy of Undetermined Significance-associated Scleromyxoedema.
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Scleromyxoedema is a rare generalized cutaneous mucinosis, which in absence of thyroid disease, occurs almost invariably in patients with monoclonal gammopathies. A 54-year-old female patient presented with complaint of tightening of skin on the extremities, abdomen, forehead, gradually progressive
Unusual Manifestations of Essential Monoclonal Gammopathy. II. Simulation of the Insulin Autoimmune Syndrome.
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In rare cases, the monoclonal immunoglobulin that characterizes essential monoclonal gammopathy interacts with a self-antigen with functional consequences and a resulting clinical syndrome. This event is presumably random and results from the clone of B lymphocytes making a monoclonal immunoglobulin
Sudden unexpected death due to a previously undiagnosed plasma cell dyscrasia.
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The plasma cell dyscrasias are a diverse group of disorders characterized by the production of a clonal paraprotein. Sudden death is a recognized complication of the plasma cell dyscrasias, most commonly in individuals with cardiac involvement by amyloidosis. However, the current forensic literature
A 19-year-old male with generalized seizures, unconsciousness and a deviation of gaze.
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Light chain deposition disease (LCDD) is a form of monoclonal immunoglobulin deposition diseases (MIDD) which in contrast to light-chain derived (AL) amyloidosis is characterized by non-congophilic, non-fibrillary monoclonal protein deposits. Systemic organ deposits are common with the kidney being
An unusual case of systemic lupus erythematosus, lupus nephritis, and transient monoclonal gammopathy.
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A 23-year-old female patient suffering from active systemic lupus erythematosus (SLE) was treated with azathioprine (2 mg/kg per day) and prednisone. Lupus nephritis class III with increasing proteinuria developed 28 months after disease onset. Treatment was switched to monthly pulse
Seizures as a Consequence of Hyperviscosity Syndrome in Two Dogs Naturally Infected with Leishmania infantum.
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Serum hyperviscosity syndrome (HVS) was documented in two dogs with canine leishmaniasis (CanL) and seizures as the major clinical complaint. In both cases, laboratory abnormalities included mild non-regenerative anemia, thrombocytopenia, hypoalbuminemia, hyperproteinemia with monoclonal gammopathy,
Restrictive cardiomyopathy in dermatomyositis.
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The association between dermatomyositis and restrictive cardiomyopathy has not been reported before. We present here the clinical, echocardiographic and muscle biopsy data for a patient with dermatomyositis and restrictive cardiomyopathy. In a 78-year-old male with a history of arterial
[Dermato-neuro syndrome during scleromyxedema: efficacy of plasmapheresis and intravenous immunoglobulin].
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BACKGROUND
Dermato-neuro syndrome is a specific neurological complication of scleromyxedema presenting with fever, coma, seizures and flu-like syndrome. To our knowledge, it has only been reported about twenty times in the literature. Its outcome is uncertain. We describe the case of a patient in
Acute Encephalitic Syndrome Induced by Scleromyxedema
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Dermato-neuro syndrome is a potentially fatal neurological complication of scleromyxedema consisting of fever, seizures, and coma. This is an overlooked scleromyxedema case of a 62-year-old female patient from 2-years ago. She was admitted to our ICU because of high fever, colloid speech, muscle
Scleromyxedema with dermato-neuro syndrome.
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Scleromyxedema is a rare connective tissue disease of unknown cause characterized by a generalized papular eruption, dermal fibroblast proliferation, and monoclonal paraproteinemia. A paroxysmal triad consisting of high fever, seizures, and coma with a flu-like prodrome can rarely occur in patients
Serum hyperviscosity syndrome associated with multiple myeloma in two cats.
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Serum hyperviscosity syndrome was diagnosed in 2 cats with multiple myeloma. Clinical signs included pale mucous membranes, dehydration, retinal hemorrhages, dilated and tortuous retinal vessels, seizures, head-tilt, nystagmus, systolic murmur, and gallop rhythm. Laboratory abnormalities included
[Monoclonal immunoglobulin (M-Ig) and skin diseases from the group of mucinoses--scleredema adultorum Buschke and scleromyxedema. Description of four cases and an overview of therapies].
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BACKGROUND
The mucinoses of the type of scleredema and scleromyxedema are diseases marked by excessive production of mucin deposits in the skin and subcutaneous tissue, which causes skin hardening. The skin and subcutaneous deposits hamper the movement of limbs, the thorax as well as mouth. The same
Multiple myeloma in sickle cell syndromes.
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Multiple myeloma (MM) is rare among patients with sickle cell syndromes (SCS). We describe six Greek sickle cell patients aged 56 to 65 years: five haemoglobin Sbeta+thalassaemia (HbSbeta+ (thal), one sickle cell anaemia (HbSS), who developed MM (three IgGkappa, one IgGlambda, one IgAkappa, and one
Does dermatoneuro syndrome have a viral aetiology?
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Scleromyxoedema is a rare disease characterized by a generalized papular and sclerodermoid cutaneous eruption. It is associated with fibroblast proliferation and mucin deposition in the dermis. Most patients have a monoclonal gammopathy, defined by the presence of IgG. Treatment of scleromyxoedema
Scleromyxedema and the dermato-neuro syndrome: case report and review of the literature.
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Scleromyxedema is a generalized form of lichen myxedematosus which is characterized by a papular and sclerodermoid skin eruption resulting from dermal fibroblast proliferation and mucin deposition. The majority of patients with scleromyxedema have a monoclonal gammopathy, and other systemic
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